BMPR2 mutation carriers | Noncarriers | p-value | |
Subjects | 23 | 21 | |
Age at diagnosis years | 28.6±12.7 | 28.5±10.4 | ns |
Age at transplant years | 35.3±12.0 | 35.0±11.5 | ns |
Sex F/M | 14/9 | 14/7 | ns |
History of haemoptysis >50 mL % | 43 | 14 | 0.034 |
mPAP at diagnosis mmHg | 63±13.4 | 64±19.5 | ns |
mPAP at last evaluation mmHg | 58±13.8 | 64±22.8 | ns |
PVR at diagnosis Wood units | 17.3±5.6 | 15.3±6.2 | 0.05 |
PVR at last evaluation Wood units | 13.2±6.3 | 16.0±5.2 | ns |
PCWP mmHg | 10.5±4.4 | 7.4±2.6 | ns |
Cardiac index at diagnosis L·min−1·m−2 | 1.97±0.6 | 2.36±0.8 | ns |
Cardiac index at last evaluation L·min−1·m−2 | 2.74±0.5 | 2.74±0.9 | ns |
DLCO % predicted | 65.1±10.7 | 66.2±10.5 | ns |
Data are presented as n or mean±sd, unless otherwise stated. ns: nonsignificant; F: female; M: male; mPAP: mean pulmonary arterial pressure; PVR: pulmonary vascular resistance; PCWP: pulmonary capillary wedge pressure; DLCO: diffusing capacity of the lung for carbon monoxide.